Colour Vision in Stargardt Disease
2011
Purpose To investigate the type and severity of colour vision deficiencies (CVDs) in Stargardt disease (STD). And, to establish how the degree of CVD relates to best-corrected visual acuity (BCVA), full field ERG (ffERG) and duration of disease.
Methods A retrospective, cross-sectional study of 97 patients with a clinical diagnosis of STD included a comprehensive medical history and a full clinical work-up, with extensive colour vision testing. Eight patients underwent anomaloscopy. ABCA4 was screened in 92 patients.
Results Patients were allocated to 5 BCVA groups and to 3 ffERG groups. Normal colour vision was found in almost 30% of patients. R/G CVDs increased as BCVA declined. More than 50% had a deutan type R/G CVD, although protan R/G CVDs became progressively apparent as BCVA decreased. A predominance of pseudoprotanomaly was evident only on anomaloscopy. Additional Blue/Yellow (B/Y) CVDs were noted in 25% of patients. B/Y CVDs and BCVA higher than 0.75 were seen in adult-onset STD. CVDs evolve to scotopization in patients with low BCVA and/or longstanding disease. Duration of disease did not correlate well with CVDs. Also, no statistically significant differences in ERG results were found between groups with or without a CVD.
Conclusion Since colour vision function is better correlated to BCVA than either disease duration or ffERG, it is a rather reliable indicator of disease severity. The presence of CVDs may help to establish an early diagnosis of STD.
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